Sickle cell syndromes are remarkable for their clinical heterogeneity, including their presentations as sudden and unexpected deaths due to a sickle cell crisis. Such cases are accompanied by various provocative factors like exertions (exercise), dehydration, hypoxia, and infections. The sudden and unexpected death of an apparently healthy baby is a tragedy for its parents, grandparents and siblings and for the paediatrician. At the same time, these deaths are challenges for the forensic and paediatric pathologists. We present here, a rare case of sudden and unexpected death in a 3-month old infant with undiagnosed sickle disease, which was not preceded by any provocating factor. We are presenting this case to emphasize that sickle cell crisis is one of the causes of sudden, unexplained infant deaths and to the highlight the role of autopsy in such cases.